tbuckley/cabot-search huggingface dataset:
AB0935 THE MIMIC THAT CAUGHT EVERYONE BY SURPRISE!
Authors: K. Kishor, P. Dogga, S. Mukherjee, A. Krishnan, A. Wakhlu
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> 57-year-old gentleman, a previous smoker, presented with a 12-month history of gradually progressive anasarca that started initially with bilateral lower limbs and involved face, abdomen and entire body over 3-4 months. Four months into his illness, the patient noticed skin changes in the form of hyperpigmentation initially over anterior chest that progressed to involve the face, bilateral upper limbs, abdomen, and lower limbs over next 3 months. It was associated with increased friability, subcutaneous oedema, and spontaneous ecchymosis. Gradually he developed progressive motor weakness, initially in both upper limbs proximally which progressed to involve distal, bilateral lower limbs and trunk with sparing of bowel and bladder functions severely limiting his activities of daily living. Over the next 15 days patient became breathless with rapidly worsening dyspnoea of exertion, dry cough and left sided pleuritic chest pain. There was history of loss of appetite and significant loss of weight 18 kgs over 4 months. General physical examination was suggestive of pallor, diffusely hyperpigmented and fragile skin over the face, neck, upper limbs, hands, back, anterior abdomen, lower limbs with multiple ecchymotic spots and subcutaneous edema with anasarca. Breath sounds were decreased bilaterally over intrascapular, axillary, infra-axillary areas and JVP elevated on Cardio-Respiratory system examination. CNS examination revealed bilateral external ophthalmoplegia with pure motor quadriparesis involving limbs and trunk without sensory, cranial nerve or autonomic involvement, Generalised wasting with asymmetric swelling over right shoulder was seen with reduced active and passive range of movements. <h3>Objectives</h3> Differentials: 1)Scleredema 2)Diffuse cutaneous systemic sclerosis <h3>Methods:</h3> Results: Final diagnosis: POEMS syndrome with primary amyloidosis. International Myeloma Working Group criteria fulfilled: Mandatory criteria- Polyneuropathy Monoclonal plasma cell proliferative disorder Atleast 1 major criteria: Elevated VEGF levels Minor criteria: Organomegaly Extravascular volume overload Skin changes Hematological involvement <h3>Conclusion</h3> The patient was started on treatment after Clinical haematology reference but died due to cardiogenic shock. This interesting case highlights POEMS as a great mimic of changes associated with various autoimmune disorders including scleroderma, SLE and vasculitis syndromes. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB0993 CONTROL OF CARDIOVASCULAR RISK FACTORS IN PATIENTS WITH SPONDYLOARTHROPATHIES VERSUS RHEUMATOID ARTHRITIS
Authors: C. Desmet, V. Ursachi, N. G. Manolache, A. Nzeusseu Toukap, M. Stoenoiu
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> It has been reported that management of cardiovascular (CV) risk factors is suboptimal in patients with rheumatoid arthritis (RA). Whether this also applies to psoriatic arthritis (PsA) or ankylosing spondylitis (AS) patients is unknown. <h3>Objectives</h3> The main objective of this study was to compare control of CV risk factors among disease groups. <h3>Methods</h3> Information on CV risk factors was collected in consecutive adult patients from rheumatology clinic, with RA, PsA or AS. Hypertension (HTN) was defined as systolic blood pressure (BP) ≥140 mmHg, diastolic BP ≥ 90 mmHg and/or antihypertensive treatment, hypercholesterolemia as LDL-cholesterol ≥ 100 mg/L and/or lipid-lowering treatment. Treatment goals were defined according to European Society of Cardiology and EULAR guidelines. <h3>Results</h3> We included 520 patients (54% women; 46% RA, 22% PsA, 32% AS). Mean age and disease duration were 52.1±14.1 and 11.0±10.6 years, respectively. Out of 520 patients, 203 (39%) had HTN, among whom 140 (69%) were treated and 86 (42%) were controlled; 293 (56%) had HC, among whom 88 (30%) were treated and 51 (17%) were controlled; 30 (6%) had type 2 diabetes, among whom 18 (60%) were controlled. Current smoking was present in 100 patients (19%), obesity in 136 patients (26%) and increased waist circumference in 192 patients (37%). Compared to RA patients, patients with spondyloarthropathies were less often in remission (40% vs. 54%, p&lt;0.005). Furthermore, patients with spondyloarthropathies differed from RA patients by a lower proportion of treated (56% vs. 84%, p&lt;0.001) and treated-to-target (24% vs. 64% p&lt;0.001) HTN, a higher proportion of active smokers (28% vs. 12%, p&lt;0.01), and lower physical activity (29% vs. 46%, p&lt;0.001). Control of LDL-cholesterol was poor in both groups (17% vs.18%, p=0.95). No significant difference between groups was found for diabetes, overweight and obesity. <h3>Conclusion</h3> Compared to patients with RA, patients with spondyloarthropathies were less well controlled both for disease activity and CV risk factors. Furthermore, lipid control was poor in both groups. If confirmed in a multicentre cohort, these results may help orienting CV prevention strategies and increasing awareness both in physicians and patients, with emphasis on spondyloarthropathies. <h3>Reference</h3> [1]Agca R et al. EULAR recommendations for cardiovascular disease risk management in patients with rheumatoid arthritis and other forms of inflammatory joint disorders: 2015/2016 update. Ann Rheum Dis. 2017;76(1):17-28. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB1173 PARAFFIN BATH REDUCES PAIN IN PATIENTS WITH HAND OSTEOARTHRITIS
Authors: F. De Azevedo, A. Souza Filho, R. Nunes, C. R. Silveira, J. E. Fonseca, F. A. Castro Rocha
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> In addition to the lack of disease modifying treatments in osteoarthritis (OA), there is also a need for safer and efficacious treatments for pain relief. Patients with hand osteoarthritis (HOA) experience pain that can be incapacitating, which is usually unresponsive to painkillers. Non-pharmacological alternatives are an interesting option for pain relief in HOA. <h3>Objectives</h3> to demonstrate that paraffin baths (PB) provide safe, significant, clinically meaningful pain relief in HOA patients. <h3>Methods</h3> (CAAE:07360819930015045) 67 patients with HOA diagnosis according to ACR criteria were prospectively recruited from March 2020 to August 2022 (interruptions due to COVID pandemic) in private rheumatology services and randomized to receive either 20 min daily sessions of PB, 3 times/week for 4 weeks or a leaflet with information for protection of hand joints (control, C). Baseline demographic, clinical and radiographic (Kellgren-Lawrence, KL score) data were registered. The evaluator was blinded to group allocation. Primary endpoint was 15% reduction of pain on movement (0-10 VAS, visual analogue scale) in the most affected joint of the hand at baseline as compared to pain value at 4w; secondary endpoints were sustained pain reduction at 12w as compared to baseline, increase in grip and pinch strength (KgF) as well as improvement in Cochin hand functional scale (CHFS), FIHOA, and SF-12 scores at 12w as compared to baseline. <h3>Results</h3> baseline characteristics of 34 paraffin and 33 control patients are shown in Table 1; three patients in each group did not return for follow up at 4 weeks; median disease duration was 58(range 3-360) months in both groups; Primary outcome was reached: baseline pain in PB (7.8±1.9) was reduced to 6.4±2.7 (p&lt;0.017) whereas baseline pain in C (7.1±1.8) was not significantly reduced at 4w (6.9±1.9; p=0.7067); reduction of pain in PB was significantly sustained at 12w (6.3±2.9) (p=0.0196). Grip and pinch strength as well as CHFS, FIHOA, and SF-12 scores were similar both at baseline and at 12 weeks in both groups. There were no major adverse events. <h3>Conclusion</h3> PB in a single-blinded prospective randomized trial provided early and sustained pain reduction in HOA. Although larger trials are needed, we believe that spreading of this cheap, easy-to-use method, should be encouraged. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB0848 DISEASE BURDEN IS ASSOCIATED WITH POOR PATIENT-REPORTED OUTCOMES IN WOMEN’S HEALTH-RELATED DOMAINS OF QUALITY OF LIFE IN SYSTEMIC SCLEROSIS: A STUDY ON 100 PATIENTS BY A MULTIDIMENSIONAL QUESTIONNAIRE
Authors: M. G. Lazzaroni, L. Moschetti, E. Pedretti, A. Lojacono, S. Zatti, F. Ramazzotto, A. Tincani, F. Franceschini, P. Airò, L. Andreoli
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Systemic Sclerosis (SSc) affects mostly women, with a significant impact on different domains of their quality of life (QoL). <h3>Objectives</h3> We aimed to identify clinical and demographic features of SSc women associated with impairment in specific QoL domains related to women's health (gynecological issues, sexual dysfunction, family planning), by means of a multidimensional ad hoc-created questionnaire [1]. <h3>Methods</h3> The questionnaire was administered to consecutive SSc female patients attending our Unit over one year (May 2021-January 2022). The Female Sexual Function Index (FSFI) questionnaire was also administered. Clinical and demographic data were retrieved from clinical charts. <h3>Results</h3> One hundred women were enrolled, with a median [IQR] age of 59 [43-67] years, median disease duration of 11 [6-17] years; 75% had limited cutaneous involvement (lcSSc); 43% had anti-centromere antibodies; 48% had received diagnosis during reproductive age (&lt;45 years). Non-regular gynecological follow-up visits were reported by 40% of patients. They were older at diagnosis and at questionnaire, more frequently post-menopausal (p:0.003, OR 4.96, 95%CI 1.68-16.6) and required immunosuppression more frequently (p:0.006, OR 2.37, 95%CI 1.02-5.50) than those regularly attending visits (Table 1). A negative impact of SSc on sexual function was reported by 60% of patients. Among the 39 patients who compiled the FSFI questionnaire, 24 (62%) displayed sexual dysfunction (score ≤26.55). These women were older and more treated (p:0.013, OR 7.50, 95%CI 1.69-33.3) as compared to patients with normal sexual function (Table 1). Among 48 patients who were diagnosed during reproductive age, 54% wished for a pregnancy after diagnosis and 30% did not reach the desired family size. These were more frequently affected by digital ulcers (p:0.040, OR 10.3, 95%CI 1.03-103) as compared to those who satisfied their family plan (Table 1). <h3>Conclusion</h3> Older age at diagnosis, digital ulcers, and immunosuppressive treatment were associated with a negative impact on women's health-related domains of QoL in SSc patients. The disease burden impairs sexual function and adherence to regular gynecological visits and receiving diagnosis during reproductive age increases the likelihood for a reduced family size. Clinicians caring for SSc women should be aware of the relevance of these domains of QoL, not usually considered in everyday practice as treatment targets. <h3>References</h3> [1]Lazzaroni M et al. Ann Rheum Dis 2022;81:1470-1. <h3>Acknowledgements</h3> GILS (Gruppo Italiano Lotta Sclerodermia) is kindly acknowledged for supporting the study with a grant. <h3>Disclosure of Interests</h3> None Declared.
AB0845 A NOVEL MARKER IN SYSTEMIC SCLEROSIS-ASSOCIATED INTERSTITIAL LUNG DISEASE: PEROXIREDOXIN-4
Authors: G. T. Alp, H. Karadeniz, I. Vasi, A. Avanoglu Guler, A. Erden, H. Küçük, Ö. Gülbahar, A. Tufan, M. A. Öztürk
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Krebs von den Lungen-6 (KL-6) and surfactant protein D (SP-D) are suggested as useful markers for the evaluation of systemic sclerosis associated interstitial lung disease(SSc-ILD)[1-3]. Increased expression of Peroxiredoxin-4(PRDX-4) mRNA had been demonstrated in the lung tissue of patients with ILD[4]. PRDX-4, a member of peroxidases family, has not been studied as a marker in SSc-ILD before. <h3>Objectives</h3> Aim of this study was to assess the clinical significance of serum PRDX-4 as a viable alternative to KL-6 and SP-D in patients with SSc-ILD. <h3>Methods</h3> The serum PRDX-4, KL-6 and SP-D levels were determined by ELISA in 61 patients with SSc(34 with ILD and 27 without ILD) and 28 healthy controls. Lung function parameters were assessed, the extent of ILD was measured by a HRCT score. <h3>Results</h3> PRDX-4 levels were significantly higher in patients with SSc-ILD than in SSc without ILD patients and controls[14.27(26.14) vs. 10.17(3.22) vs. 9.68(2.06)ng/ml, median,(IQR), p=0.001). KL-6 levels were also remarkbly different between groups[134.25(224.28) vs. 103.90(34.81) vs. 92.84(29.16) U/ml, median,(IQR), p=0.002)]. Moreover PRDX-4, KL-6 and SP-D levels in patients with progressive ILD were significantly higher in stable ILD patients(p&lt;0.001). PRDX-4, KL-6 and SP-D were correlated each other(p&lt;0.01). Receiver operator characteristic curve analysis demonstrated high sensitivity and specificity of PRDX-4 for the determination of SSc-ILD and progressive pulmonary fibrosis. (AUC:0.705, p&lt;0.01 and AUC:0.936, p&lt;0.001). <h3>Conclusion</h3> PRDX-4 is more sensitive and spesific than KL-6 and SP-D and it is a potentially useful marker in assessing interstitial lung disease and progressive pulmonary fibrosis in patients with SSc. <h3>References</h3> [1]Kumánovics, G., et al., Comprehensive investigation of novel serum markers of pulmonary fibrosis associated with systemic sclerosis and dermato/polymyositis. Clin Exp Rheumatol, 2008. 26(3): p. 414-20. [2]Yanaba, K., et al., Comparative study of serum surfactant protein-D and KL-6 concentrations in patients with systemic sclerosis as markers for monitoring the activity of pulmonary fibrosis. J Rheumatol, 2004. 31(6): p. 1112-20. [3]Yamane, K., et al., Serum levels of KL-6 as a useful marker for evaluating pulmonary fibrosis in patients with systemic sclerosis. J Rheumatol, 2000. 27(4): p. 930-4. [4]Elko, E.A., et al., Peroxiredoxins and beyond; redox systems regulating lung physiology and disease. Antioxidants &amp; Redox Signaling, 2019. 31(14): p. 1070-1091. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB0827 CLINICAL SIGNIFICANCE OF SERUM FERRITIN IN PATIENTS WITH SYSTEMIC SCLEROSIS
Authors: S. Markovich Sholomon, Y. Braun-Moscovici, A. Balbir-Gurman
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> The main systemic sclerosis (SSc) manifestations are skin thickening, microangiopathy and ischemic changes in tissues, fibrotic damage to the lungs, heart, kidneys, and digestive system, arthritis, and myopathy. Acute phase reactants (APR) like erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) reflect inflammation activity in various inflammatory conditions. Ferritin is a protein bound to iron; low serum ferritin indicates iron deficiency and/or anemia. Instead, high ferritin levels are associated with inflammatory and non-inflammatory conditions such as dermatomyositis, pulmonary fibrosis, lupus, systemic COVID-19, vasculitis, tissue damage, thromboembolic complications, and metastatic cancer. The possible role of ferritin in SSc as APR is unclear. <h3>Objectives</h3> We aimed to assess whether ferritin levels can reflect the severity of SSc and predict the outcome. <h3>Methods</h3> 241 files of SSc patients with information on serum ferritin level (ferritin over 300 mg/dL is considered elevated) who visited the Rambam Rheumatology Institute in the years 2004-2021were used for retrospective analysis. Patients' demographic, clinical, laboratory, imaging, and respiratory function data were collected from electronic hospital files. Statistics included Student's T-test, Pearson's chi-squared test, and Kaplan-Meier curve; statistical significance was determined as p&lt;0.05. <h3>Results</h3> 36 patients (FerEl-SSc) had elevated ferritin values; the rest (n=205) represented the second group (FerNor-SSc). Significant differences were seen in gender (male 44.4% - 15.6%), disease duration (4.56 - 7.7 years), modified Rodnan skin score (12.3 - 6.9), as well as in incidence of lung (65.7% - 38.7%), heart (51.4% - 21.1%), and renal (28.6% - 5.9%) involvement. Increased ferritin correlated with elevated ESR, CRP, creatinine, creatine kinase, troponin, and reduced hemoglobin, impaired pulmonary function tests and reduced left ventricular ejection fraction on echocardiography. Patients with elevated ferritin had a significant increase in mortality rates (52.8% and 35.1%) and non-significant reduction in survival. <h3>Conclusion</h3> Our study demonstrated that ferritin has a potential as a sensitive marker for SSc severity in term of skin thickening, vital organ complications, and mortality. The ferritin test is simple and inexpensive, it can add to the complex SSc assessment and contribute to treatment decision-making in complicated SSc. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB0802 PRESENCE OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS IN PATIENTS WITH NASAL POLYPS AND ASTHMA. STUDY OF A SINGLE REFERRAL CENTER
Authors: F. Benavides-Villanueva, J. Loricera, V. Calvo-Río, R. Blanco
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Eosinophilic granulomatosis with polyangiitis (EGPA) is included in anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). It mainly affects patients with asthma, sinusitis, allergic rhinitis, and nasal polyposis. <h3>Objectives</h3> To assess <b>a</b>) prevalence of clinical features of EGPA in patients with clinical history of nasal polyps and asthma, and <b>b</b>) proportion of positive ANCA (+ANCA) test in those patients. <h3>Methods</h3> Retrospective study of patients with clinical history of asthma and nasal polyps with histologic study from a referral center between 2021 and 2022. The measurement of ANCA were performed by chemiluminescence assay using IO-FLASH (Inova, San Diego, CA). Diagnosis of EGPA was based on ACR/EULAR 2022 criteria <b>[1].</b> Disease activity was assessed with Birmingham Vasculitis Activity Score (BVAS). All biopsies of nasal polyps were examined by the same expert pathologist to assess the presence of eosinophil aggregates. <h3>Results</h3> A total of 123 biopsies with eosinophil aggregates were studied between 2021 and 2022. Twenty-three patients (19%) had history of asthma. Of them, we objectified EGPA criteria in 12 patients (10% of the series of 123 patients). General features of these 12 patients are summarized in <b>Table</b>. Seven patients had more than 20 eosinophil aggregates per field in polyp biopsy. The median [IQR] BVAS score was 0[0-0]; just 2 patients showed activity measured by BVAS score (with a mean of 3 points). ANCA test was positive in 1 (8.3%) patient. <h3>Conclusion</h3> According to data from our series, approximately 10% of patients with nasal polyps with biopsy meet EGPA criteria. <h3>References</h3> [1]Grayson PC, et al. Arthritis and Rheumatology 2022;74:386–392. PMDI: 35106968. [2]Brescia G, et al. Am J Otolaryngol. 2020;41. PMDI: 32810787 [3]Contro G, et al. <i>Clin Rheumatol</i>. 2021;40:1949–1957. PMDI: 33094393 <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> Fabricio Benavides-Villanueva: None declared, J. Loricera Speakers bureau: Dr. J. Loricera had participation in company-sponsored speaker´s bureau from Roche, Novartis, Galápagos, UCB Pharma, MSD, Celgene, and Grünenthal and received support for attending meetings and/or travel from Janssen, Abbvie, Roche, Novartis, MSD, UCB Pharma, Celgene, Lilly, Pfizer, Galápagos., Vanesa Calvo-Río: None declared, Ricardo Blanco Speakers bureau: Speakers bureau: Abbvie, Pfizer, Roche, lilly, Bristol-Myers, Janssen, Galapagos and MSD., Consultant of: Consultant of: Abbvie, Pfizer, Roche, lilly, Bristol-Myers, Janssen and MSD, Grant/research support from: Grant/research support from: Abbvie, MSD, novartis and Roche.
AB0769 CARDIAC AND PULMONARY INVOLVEMENTS IN BEHCET’S SYNDROME: A CASE SERIES OF 93 PATIENTS
Authors: S. Chadli, H. Khibri, M. Bourkia, N. Moatassim, W. Ammouri, M. Maamar, H. Harmouche, M. Adnaoui, Z. Tazi Mezalek
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Behçet syndrome (BS) is a multisystemic vasculitis of unknown etiology, frequently observed in the Mediterranean region and the Middle east. Pulmonary involvement (PI) and cardiac involvement (CI) are rarely described in BS, with a prevalence of less than 5%. However, they are entailed to high morbidity and mortality. <h3>Objectives</h3> Our work aims to describe the profile and outcomes of BS patients presenting with cardiopulmonary lesions. <h3>Methods</h3> We retrospectively conducted a descriptive and monocentric study, between 2020 and 2022, including 576 BS patients. <h3>Results</h3> 93 patients were enrolled (16%). The sex ratio (M/F) was 4.8 and the mean age was 3 ± 6 years [19-51]. Cardiovascular risk factors comprised smoking (26%), high blood pressure (10%), and diabetes (9%). Patients were often asymptomatic (78%) or presented with chest pain (23%), dyspnea (11.5%), dry cough (5%), and hemoptysis (5%). On chest CT angiography, we observed pulmonary artery aneurysms (70%), pulmonary artery thrombosis (24%), and alveolar hemorrhage (1%). Cardiac involvement (46%) included right intracardiac thrombosis (24%), pericarditis (10%), coronary thrombosis (4%), myocarditis (1%), and endomyocardial fibrosis (1%). Deep vein thrombosis was reported (28%) in the superior vena cava (14%), inferior vena cava (11%), and lower extremities (8%). Extravascular involvements were mostly mucocutaneous (79%), ocular (40%), articular (31%), and neurological (15%). Patients were treated with colchicine (83%), glucocorticoids (92%), curative anticoagulation (77%), cyclophosphamide (42%), azathioprine (36%), Infliximab (8%) and ciclosporin (1%). Surgery was performed for 8% of the patients. Relapse and death were recorded in respectively 15% and 7% of the cases. <h3>Conclusion</h3> PI and CI are serious complications of BS, often lately discovered and underdiagnosed, due to their discrete evolution. Systematic screening should thus be considered in at-risk patients, especially young males presenting with mucocutaneous lesions and venous thrombosis. Prompt and adequate immunosuppressive therapy is usually linked to favorable outcomes. <h3>References</h3> [1]Alfredo Nicodemos Cruz Santana, Telma Antunes, Juliana Monteiro de Barros, et al. Pulmonary involvement in Behcet's disease: a positive single-center experience with the use of immunosuppressive therapy. J Bras Pmeumol. 2008. 34;362-6. [2]Selami Demirelli, Husnu Degirmenci, Sinan Inci, et al. Cardiac manifestations in Behcet's disease. Intractable Rare Dis Res. 2015. 4(2);70-5. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.